All eukaryotic cells contain specialized organs called mitochondria that produce energy and house a host of metabolic processes essential for life. To achieve a fully functional state, mitochondria require proteins synthesized in the cell's cytosol to be imported into the proper location on or within them. This process is complicated because each mitochondrion is composed of two distinct compartments arising from the set of lipid membranes that surround them. Genetic, biochemical, and cellular studies have identified a complex translocation system, including translocons on the mitochondrial outer and inner membranes and intermembrane space of the mitochondrial FIG. 26.
The outer membrane contains the TOM (translocon of the outer membrane) protein complex, whereas the inner membrane contains the TIM23 (translocase of the inner membrane) and TIM22 complexes, which differ in their substrate specificity. Defects in the TIM22 import pathway lead to an inherited disease called deafness-dystonia syndrome, in which patients have deafness, blindness, and dystonia.
There is a need for compounds that are specific inhibitors of mitochondrial protein translocation. The inhibitors modulate the assembly or function of mitochondria.
There is a need for compounds effective for a disorder related to mitochondrial protein translocation.
The embodiments below address the above identified needs and issues.